WOFAPS 2025 8th World Congress of Pediatric Surgery

Ampaipan Boonthai ¹, Paul Losty ¹, Songpon Ketsuwan ²

¹ Division of Pediatric Surgery, Department of Surgery, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
² Division of Gastroenterology, Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand

Purpose

Biliary atresia (BA) is a progressive fibro-obliterative cholangiopathy affecting newborns and requires timely operative management to prevent fatal liver failure. This study outlines a comprehensive management strategy programme evaluating contemporary outcome(s) of biliary atresia patients treated at a national liver transplant centre.

Methods

Medical case records of BA patients undergoing Kasai portoenterostomy (KPE) at a liver transplant center between 2000-2022 were analysed. Demographics, clinical presentation, diagnosis and operative treatment(s) were comprehensively studied. Outcome metrics examining those who underwent KPE before and after 60 days of age were compared. Primary outcomes measured included ( i ) native liver survival, ( ii ) patients being listed for liver transplantation and ( iii ) survival with a liver transplant.

Results

Twenty four BA patients (45.8% Male vs 54.2% Female) are included in this preliminary report. 13/24(54.2%) BA index cases presented directly at outpatient clinics while 11/24 (45.8%) were transferred from other hospital centres with no difference(s) noted in the timing of clinical presentation between these two distinct groups (73 ±29 vs 69±16 days p=0.663). Initial ultrasonography exams were performed in 16/24 (66.7%) patients with an atretic gall bladder being the most common finding recorded in 53% cases followed by a triangular cord sign in 26% patients. All patients had DISIDA imaging with no excretion observed. Mean age at KPE operation was 61.4 days. Bridging fibrosis was observed from liver biopsies in 11/24 (46%) patients without notable difference(s) in patients having KPE before 60 days (67% vs 43%; p = 0.43). 66.7% patients who had Kasai operation before 60 days of age survive on native liver vs 41.2% having KPE after 60 days of age (p = 0.57) Liver transplantation was performed in 45% of BA patients with a transplant mean ‘ waiting time ’ after listing of 42±24 months. Ten-year and 20-year overall survival (OS) for biliary atresia patients at this national centre were 95% and 89% respectively.

Conclusion

This study demonstrates superior outcomes achieved at a liver transplant center for children with biliary atresia. Centralisation of biliary atresia management at specialist liver centers with transplantation capacity best optimize outcome(s) of this disease.